Pediatric spinal ependymomas: an unpredictable and puzzling disease. Long-term follow-up of a single consecutive institutional series of ten patients

Abstract

MethodsTen consecutive children (0–18 years) who underwent primary tumor resection for a spinal ependymoma between 1980 and 2011 were included in this retrospective study. Gross motor function and activities of daily life were scored according to the Barthel Index.ConclusionThree out of six pediatric patients treated for spinal myxopapillary ependymoma are disease-free after 11 to 33 years of follow-up. The other three have progressive disease, after relapses occurring after 4.5, 7, and 20 years, respectively. One out of two patients with grade II ependymoma had progressive disease from 10 years after initial surgery but is in full-time work in spite of widespread metastatic disease after 32 years. One of the two children with grade III tumor died from progressive disease 17 years from primary diagnosis, while the last one is tumor-free after 19 years. The quality of life is good for three of the four patients with widespread disease, and they are managed conservatively aiming at symptomatic treatment intervention if necessary. We strongly advocate lifelong follow-up for children treated for spinal ependymomas.

Posterior fossa ependymoma in childhood: 60 years event-free survival after partial resection—a case report

“…Most probably, his residual tumor has been present ever since the operation in 1954. The situation therefore seems stable and resembles the puzzling situation we have observed in a small group of pediatric patients with recurrent disease decades after primary treatment for distal spinal ependymoma [ 5 ].…”

“…Ependymoma is the third most common posterior fossa tumor in childhood after astrocytoma and medulloblastoma/PNET [ 1 , 2 ]. A spinal primary location is exceedingly rare in children, as opposed to adults, where a spinal presentation is the most common of all ependymoma locations [ 5 ]. Although there is anecdotal evidence that some patients can be successfully treated with surgery only following gross total resection (GTR), the role of postoperative radiation has clearly been established in retrospective studies and prospective protocols.…”

Radiation therapy after subtotal resection of pediatric grade II/III spinal ependymomas: what is the evidence?

“…However, the role of RT after STR is controversial. We reviewed the literature on pediatric patients with grade II/III spinal ependymoma and found 13 cases that were subtotally resected (Table 1) [4,[6][7][8][9][10][11][12]. The median age was 9 (range 1 to 20).…”

An integrative analysis of treatment, outcomes and prognostic factors for primary spinal anaplastic ependymomas

“…The goal of our study was to perform an integrative analysis of the individual patient data, therefore, aggregated data sets, in which individual patient data was grouped, were not included in the analysis. Finally, we identified 23 articles with a total of 40 patients who met our criteria [2][3][4][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25]. The number of patients included from each study varied from one to 14 …”